Developmental and Immune-mediated Mucocutaneous Conditions - part I COPY

Question 1

categories of mucocutaneous conditions

Answer 1

1. developmental

2. immune-mediated

Question 2

developmental mucocutaneous conditions

Answer 2

1. ectodermal dysplasia
2. white sponge nevus
3. Peutz-Jeghers syndrome
4. Hereditary hemorrhagic telangiectasia

Question 3

immune-mediated mucocutaneous conditions

Answer 3

1. pemphigus vulgaris
2. mucous membrane pemphigoid
3. bullous pemphigoid
4. erythema multiforme
5. erythema migrans (geographic tongue)
6. linchen planus
7. lichenoid mucositis
8. lupus erthematosus
9. systemic sclerosis
10. CREST syndrome

Question 4

etiology of pemphigus vulgaris (PV)

Answer 4

autoimmune

Question 5

Pemphigus vulgaris (PV) is due to inappropriate production of what?

Answer 5

antibodies by the host directed against host tissue (autoantibodies)…

damage to host by host’s own immune response

Question 6

Pemphigus vulgaris (PV) autoantibodies destroy what?

Answer 6

desmosomes

Question 7

role of desmosomes

Answer 7

bond epithelial cells together

Question 8

what happens when antibodies inhibit desmosomes?

Answer 8

inhibits adherence so a split develops in the epithelium

Question 9

T/F: Pemphigus vulgaris (PV) is relatively rare

Answer 9

true, ~5 cases per million dx’d each year in general population

Question 10

what is the average age of pts dx’d with pemphigus vulgaris (PV)?

Answer 10

50 y.o.

Question 11

what is the gender predilection for pemphigus vulgaris (PV)?

Answer 11

no gender predilection

Question 12

oral lesions in pts with pemphigus vulgaris (PV)

Answer 12

1. superficial, ragged erosions and ulcerations

2. any mucosal surface

Question 13

T/F: the oral lesions in pts with pemphigus vulgaris (PV) are the “first to show, last to go”

Answer 13

true, oral lesions are the initial manifestation of the disease and the most difficult to resolve with treatment

Question 14

clinical features of pemphigus vulgaris (PV)

Answer 14

1. flaccid vesicles, bullae on skin

2. + Nikolsky sign

Question 15

+ Nikolsky sign

Answer 15

inducing a bulla by applying firm, lateral pressure to normal appearing skin

Question 16

what is rarely seen intact intraorally in pts with pemphigus vulgaris (PV)?

Answer 16

flaccid vesicles, bullae

Question 17

what is important in managing pemphigus vulgaris (PV)?

Answer 17

plaque control

Question 18

normal tissue adjacent to ulceration or erosion of pemphigus vulgaris (PV) should be sampled for what?

Answer 18

1. direct immunoflorescent (in Michel’s solution)

2. light microscopic evaluation

Question 19

histo of pemphigus vulgaris (PV)

Answer 19

1. INTRAEPITHELIAL clefting above the basal layer (i.e. within the epithelium)
2. acantholysis

Question 20

acantholysis

Answer 20

breakdown of spinous layer; cells appear to fall apart

Question 21

what remains attached to the CT seen microscopically in pemphigus vulgaris (PV)?

Answer 21

only basal cell layer of epithelium

Question 22

immunopathology identifies what?

Answer 22

immune responses associated with diseases

Question 23

immunofluorescence

Answer 23

technique that uses fluorescent-labeled antibodies to detect specific targets

Question 24

T/F: immunopathology is used to help identify several immune-mediated conditions

Answer 24

true

Question 25

direct immunofluorescence (DIF) is used to detect what?

Answer 25

autoantibodies bound to pt's tissues

Question 26

indirect immunofluorescence (IIF) is used to detect what?

Answer 26

antibodies circulating in the blood

Question 27

immuno features of pemphigus vulgaris (PV)

Answer 27

1. both DIF and IIF studies will be positive in pemphigus vulgaris 2. autoantibodies bind desmosomal components (desmoglein 1 & 3)

Question 28

DIF-immunoreactants are what in pemphigus vulgaris (PV)?

Answer 28

in intercellular areas b/w the surface epithelial cells "chicken wire" with SUPRAbasilar cleft

Question 29

tx of pemphigus vulgaris (PV)

Answer 29

systemic corticosteroids - often azathiprine or other steroid-sparing agents

Question 30

why does topical corticosteroids have little effect in tx'ing pemphigus vulgaris (PV)?

Answer 30

PV is a systemic disease

Question 31

what percent of pemphigus vulgaris (PV) cases will resolve on their own after 10 years?

Answer 31

~30%

Question 32

T/F: pemphigus vulgaris (PV) is usually fatal if not tx'd

Answer 32

true

Question 33

why may pemphigus vulgaris (PV) be fatal if not tx'd?

Answer 33

1. severe infection 2. loss of fluids/electrolytes 3. malnutrition due to mouth pain

Question 34

prior to corticosteroid therapy, what mortality rates of pemphigus vulgaris (PV) cases were what percent?

Answer 34

60-90%

Question 35

what may lead to mortality of pts with pemphigus vulgaris (PV)?

Answer 35

complications of long-term steroid

Question 36

what is the morality rate of pts with pemphigus vulgaris (PV) today?

Answer 36

5-10% mortality

Question 37

mortality today in pts with pemphigus vulgaris (PV) is usually due to what?

Answer 37

complications of therapy (side effect of steroids, immune-suppression)

Question 38

what drug is used to tx pemphigus vulgaris (PV) suppresses bone marrow and is a carcinogen?

Answer 38

azathioprine